Raynaud's phenomenon is a symptom complex manifested as intermittent fingertip ischemia caused by cold or other sympathetic drivers. Secondary Raynaud's phenomenon is often more severe and could even lead to finger ulceration, making it particularly complicated to treat. We describe a case of severe Raynaud's phenomenon secondary to subclinical hypothyroidism lasting for more than 6 hours in a 65-year-old woman. The patient was also diagnosed with hypothyroidism, epilepsy, and secondary soft tissue infection of the right middle and ring fingers. After careful multidisciplinary consultation and discussion, the patient received vasodilation, anticoagulation, thyroxine supplementation, stellate ganglion block, hyperbaric oxygen therapy and debridement. The patient responded well to the medication, avoiding amputation or obviously dysfunction. Multidisciplinary team gathering the doctors from different departments proposes appropriate strategies for patients with severe Raynaud's phenomenon and could improve the prognosis and satisfaction of patient effectively.
Female ; Humans ; Aged ; Hypothyroidism/complications* ; Raynaud Disease/diagnosis*

OBJECTIVES: To investigate the clinical findings upon initial diagnosis and extraglandular manifestations in Korean patients with primary Sjogren's syndrome (pSS). METHODS: We collected clinical and laboratory data from 238 pSS patients enrolled at Seoul National University Hospital, Seoul National University Bundang Hospital and Seoul Medical Center from March 2011 to December 2014. All patients met the American-European Consensus Group criteria for pSS. RESULTS: Upon initial diagnosis, sicca symptoms (xerophthalmia or xerostomia) as the chief complaint were only observed in 129 (54.2%) pSS patients, while extraglandular manifestation was more common as the chief complaint in male patients or those with younger age (<40 years) than female or older patients (both p<0.05). Extraglandular manifestations were found in 178 (74.8%) patients, with musculoskeletal manifestations being most common (53.8%). Peripheral neuropathy in pSS patients was associated with Raynaud phenomenon and elevated serum total immunoglobulin G (IgG) levels (both p<0.05). Serum beta2-microglobulin (beta2-M) levels were significantly correlated with European League against Rheumatism (EULAR) Sjogren's syndrome disease activity index, erythrocyte sedimentation rate and serum total IgG (all p<0.001), and were higher in patients with extraglandular manifestations than those without (p<0.05). Serum C3 levels were decreased in patients with extraglandular manifestation, compared to those without (p<0.05). Malignant lymphoma was found in Korean pSS patients (1.7%) and associated with elevated serum beta2-M levels (p<0.0001). CONCLUSION: Extraglandular manifestations were common in pSS patients and may be a diagnostic tool for male or younger pSS patients. Serum beta2-M levels can be useful markers for monitoring pSS patients.
beta 2-Microglobulin ; Consensus ; Diagnosis ; Erythrocyte Indices ; Female ; Humans ; Immunoglobulin G ; Lymphoma ; Male ; Peripheral Nervous System Diseases ; Raynaud Disease ; Rheumatic Diseases ; Seoul ; Sjogren's Syndrome*

BACKGROUND/AIMS: We investigated whether transthoracic echocardiography-suspected pulmonary hypertension (PH) affects survival in systemic lupus erythematosus (SLE) patients and examined factors associated with PH occurrence and survival. METHODS: This retrospective single-center study included 154 Korean SLE patients fulfilling the American College of Rheumatology criteria (January 1995 to June 2013). Student t test, Mann-Whitney U test, Kaplan-Meier curves, and log-rank tests were used for comparisons. RESULTS: A total of 35 SLE patients with PH (SLE/PH+) and 119 without PH (SLE/PH-) were analyzed. Higher percentages of interstitial lung disease, Raynaud's phenomenon (RP), World Health Organization functional classification III/IV, and cardiomegaly were found in SLE/PH+ compared to SLE/PH-. Furthermore, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was significantly higher in SLE/PH+ (2.46 +/- 1.245 vs. 1.00 +/- 1.235), whereas survival rates were significantly higher in SLE/PH- in log-rank tests (p = 0.001). In multivariate analysis, the adjusted mortality hazard ratio (HR) for SLE/PH+ patients was 3.10. Subgroup analysis demonstrated a higher percentage of lupus nephritis in the SLE/PH+ patients who died (p = 0.039) and low complement-3 levels (p = 0.007). In univariate analysis, the mortality HR for SLE/PH+ patients with lupus nephritis was 4.62, whereas the presence of RP decreased the mortality risk in multivariate analysis; adjusted HR, 0.10. CONCLUSIONS: PH is an independent factor predicting survival in SLE patients. The presence of lupus nephritis resulted in an increased trend for mortality, whereas coexistence of RP was associated with a better survival prognosis in SLE/PH+ patients.
Adolescent ; Adult ; Cardiomegaly/diagnosis/epidemiology ; Chi-Square Distribution ; Female ; Humans ; Hypertension, Pulmonary/diagnosis/*mortality ; Kaplan-Meier Estimate ; Lung Diseases, Interstitial/diagnosis/mortality ; Lupus Erythematosus, Systemic/diagnosis/*mortality ; Lupus Nephritis/diagnosis/mortality ; Male ; Multivariate Analysis ; Prognosis ; Proportional Hazards Models ; Raynaud Disease/diagnosis/epidemiology ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Young Adult

Raynaud's phenomenon (RP) is characterized by reversible digital vasospasm triggered by exposure to cold or emotional stress. It manifests with unique 'triphasic' (pallor, cyanosis, erythema) or 'biphasic' (white, blue) color change. Clinically, RP is classified as primary RP, which does not have an underlying associated cause with a relatively benign course, and secondary RP, which predates various cause such as systemic connective tissue disease (CTD). Therefore, RP must be differentiated for detection of emerging CTD such as systemic sclerosis (SSc), systemic lupus erythematosus, and mixed conective tissue disease, ect. Nailfold capillaroscopy (NFC) is a non-invasive morphological study used routinely with antinuclear antibodies for parallel of secondary RP. A recent study showed NFC to be the best predictor of transition from a primary RP to secondary RP. The well-established role of NFC for early diagnosis of SSc, and potential for monitoring disease progression and predictive clinical complication makes NFC an important tool in research and clinical practice. In this paper, I present a recent update with emphasis on its possible role as a reliable diagnostic tool and biomarker in secondary RP.
Antibodies, Antinuclear ; Connective Tissue Diseases ; Cyanosis ; Disease Progression ; Early Diagnosis ; Humans ; Lupus Erythematosus, Systemic ; Microscopic Angioscopy* ; Raynaud Disease ; Scleroderma, Systemic ; Stress, Psychological

PURPOSE: To compare the thickness of the lamina cribrosa (LC) and vascular factors of early normal-tension glaucoma (NTG) patients with high and low intraocular pressure (IOP) that are expected to be associated with the development of glaucoma. METHODS: Seventy-one Korean NTG patients with low IOP (the highest IOP <15 mmHg, 40 patients) and high IOP (the lowest IOP >15 mmHg, 31 patients) were included in this study. The thickness of LC and vascular factors were compared. The thickness of the LC was measured using the enhanced depth imaging method with spectral domain optical coherence tomography (Heidelberg Spectralis). RESULTS: The mean thickness of the central LC was 190.0 +/- 19.2 microm in the low IOP group and 197.8 +/- 23.6 microm in the high IOP group, but there was no statistical significant difference between the two groups (p > 0.05). The prevalence of self-reported Raynaud phenomenon was significantly higher in the low IOP group (33.0%) than the high IOP group (10.3%, p = 0.04). CONCLUSIONS: The laminar thickness did not significantly differ between the high and low IOP groups. However, the prevalence of Raynaud phenomenon was higher in the low IOP groups. These results suggest that the development of glaucoma with low IOP patients may be more influenced by peripheral vasospasm, such as Raynaud phenomenon, rather than laminar thickness in NTG.
Aged ; Cross-Sectional Studies ; Female ; Humans ; *Intraocular Pressure ; Low Tension Glaucoma/*diagnosis ; Male ; Middle Aged ; Nerve Fibers/pathology ; Optic Disk/*pathology ; Optic Nerve Diseases/*diagnosis ; Raynaud Disease/*diagnosis ; Retinal Ganglion Cells/pathology ; Tomography, Optical Coherence ; Tonometry, Ocular ; Vision Disorders/diagnosis ; Visual Fields

The aim of this study was to determine whether skin temperature measurement by digital thermography on hands and feet is useful for diagnosis of Raynaud's phenomenon (RP). Fifty-seven patients with RP (primary RP, n = 33; secondary RP, n = 24) and 146 healthy volunteers were recruited. After acclimation to room temperature for 30 min, thermal imaging of palmar aspect of hands and dorsal aspect of feet were taken. Temperature differences between palm (center) and the coolest finger and temperature differences between foot dorsum (center) and first toe significantly differed between patients and controls. The area under curve analysis showed that temperature difference of the coolest finger (cutoff value: 2.2degrees C) differentiated RP patients from controls (sensitivity/specificity: 67/60%, respectively). Temperature differences of first toe (cutoff value: 3.11degrees C) also discriminated RP patients (sensitivity/specificity: about 73/66%, respectively). A combination of thermographic assessment of the coolest finger and first toe was highly effective in men (sensitivity/specificity : about 88/60%, respectively) while thermographic assessment of first toe was solely sufficient for women (sensitivity/specificity: about 74/68%, respectively). Thermographic assessment of the coolest finger and first toe is useful for diagnosing RP. In women, thermography of first toe is highly recommended.
Adult ; Diagnosis, Differential ; Female ; Fingers/*physiology ; Humans ; Male ; Middle Aged ; ROC Curve ; Raynaud Disease/*diagnosis ; Sensitivity and Specificity ; Skin Temperature ; *Thermography ; Toes/*physiology

Although it is difficult to reach a diagnosis in patients who complain of pain or sensitivity to cold in their hands, Raynaud's phenomenon is most often suspected in such cases. Symptoms of Raynaud's phenomenon include pallor, cyanosis, and redness following cold exposure. Glomus tumors can also increase patients' sensitivity to cold. In this case, our patient complained of symptoms indicative of Raynaud's phenomenon. Although treatment of Raynaud's phenomenon improved the symptoms, pain persisted in the fourth finger of the left hand. We diagnosed the patient with a glomus tumor and, after surgical treatment, the patient's symptoms improved. Here, we have also reviewed and discussed a number of reports of glomus tumors associated with Raynaud's disease. Diagnosing a glomus tumor in the hand may take some time, and the diagnosis could be further delayed if accompanied by Raynaud's phenomenon. Although glomus tumors are rare, we suggest that clinical awareness is important for early diagnosis and treatment.
Cyanosis ; Diagnosis ; Early Diagnosis ; Fingers ; Glomus Tumor* ; Hand* ; Humans ; Pallor ; Raynaud Disease*

Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, lungs, heart, gastrointestinal tract, kidneys, and musculoskeletal system. Although up to 90% of patients with scleroderma have been estimated to have gastrointestinal involvement, liver disease has been reported only rarely. A 51-year-old woman was hospitalized due to esophageal variceal bleeding. Her serum was positive for anti-nuclear antibody and anti-centromere antibody. Sclerodactyly was noted on both hands, and she had recently developed Raynaud's syndrome. Punch biopsy of the hand showed hyperkeratosis, regular acanthosis, and increased basal pigmentation in the epidermis, and thick pale collagenous bundles in the dermis. Liver biopsy showed chronic active hepatitis with bridging fibrosis. Consequently, she was diagnosed with liver cirrhosis due to autoimmune hepatitis (AIH) combined with SSc. AIH had subsided after administration of prednisolone at 40 mg per day. She received 5-10 mg/day of prednisolone as an outpatient, and her condition has remained stable. Patients with either AIH or SSc should be monitored for further development of concurrent autoimmune diseases. The early diagnosis of AIH combined with SSc will be helpful in achieving optimal management.
Anti-Inflammatory Agents/therapeutic use ; Antibodies, Antinuclear/blood ; Esophageal and Gastric Varices ; Female ; Gastrointestinal Hemorrhage ; Hepatitis, Autoimmune/complications/*diagnosis/drug therapy ; Humans ; Liver Cirrhosis/*diagnosis/etiology/pathology ; Middle Aged ; Prednisolone/therapeutic use ; Raynaud Disease/diagnosis ; Scleroderma, Systemic/complications/*diagnosis ; Skin/pathology

OBJECTIVETo investigate the clinical manifestations, diagnosis and interventions of pulmonary hypertension (PAH) in patients with systemic lupus erythematosus (SLE).

METHODSFrom January 2001 to December 2007, 798 SLE patients without prior diagnosis were admitted in our hospital, among whom 39 were identified to have concomitant PAH defined by echocardiography. The clinical data of the 39 cases were analyzed retrospectively.

RESULTSThe incidence of PAH was 4.9% in these cohort of SLE patients. The 39 SLE patients with concomitant PAH included 5 men and 34 women with a mean age of 34-/+12 years. Positive correlations were found between the occurrence of PAH and the Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia (P < 0.05). Patients with higher scores for SLE Disease Activity Index were liable to PAH. The presence of Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia is correlated to greater severity PAH with poor prognosis.

CONCLUSIONPAH is not a rare concomitant disease in SLE patients. The presence of Raynaud phenomenon, fingertip vasculitis, anti-u1RNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia all suggest the likeliness of PAH in SLE patients, and echocardiographic examination may help derive an early diagnosis.

Adolescent ; Adult ; Aged ; China ; epidemiology ; Early Diagnosis ; Echocardiography ; Female ; Humans ; Hypertension, Pulmonary ; complications ; diagnosis ; epidemiology ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; Male ; Middle Aged ; Raynaud Disease ; complications ; Retrospective Studies

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibodies and immune complex depositions, has been known that it could be aggravated during pregnancy. We report a case who developed massive pericardial effusion, pleural effusion, and proteinuria during the second trimester of pregnancy. This patient had a history of leukopenia for several years and showed several features of SLE before pregnancy, however, no specific diagnosis was made before pregnancy. High dose oral glucocorticoid with intravenous immunoglobulin administration was done, but serositis and nephritis did not improve. The induced abortion and the use of immunosuppresive agent resulted in remission. We emphasize the importance of the early diagnosis and management of SLE before pregnancy in the patients with the features of autoimmune disease such as leukopenia, skin lesion and Raynaud phenomenon for successful outcome.
Abortion, Induced ; Antigen-Antibody Complex ; Autoantibodies ; Autoimmune Diseases ; Diagnosis ; Early Diagnosis ; Female ; Humans ; Immunoglobulins ; Leukopenia ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Nephritis* ; Pericardial Effusion ; Pleural Effusion ; Pregnancy Trimester, Second ; Pregnancy* ; Proteinuria ; Raynaud Disease ; Serositis* ; Skin